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首页> 外文期刊>Developmental Immunology: Journal of Immunology Research >Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Current Status and Future Direction
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Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Current Status and Future Direction

机译:系统性红斑狼疮的肺动脉高压:现状和未来方向。

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Pulmonary arterial hypertension (PAH) is commonly associated with connective tissue diseases (CTDs) including systemic sclerosis and systemic lupus erythematosus (SLE). The prevalence of PAH in SLE is estimated to be 0.5% to 17.5%. The pathophysiology of PAH involves multiple mechanisms from vasculitis andin-situthrombosis to interstitial pulmonary fibrosis which increases pulmonary vascular resistance, potentially leading to right heart failure. Immune and inflammatory mechanisms may play a significant role in the pathogenesis or progression of PAH in patients with CTDs, establishing a role for anti-inflammatory and immunosuppressive therapies. The leading predictors of PAH in SLE are Raynaud phenomenon, anti-U1RNP antibody, and anticardiolipin antibody positivity. The first-line of diagnostic testing for patients with suspected SLE-associated PAH (SLE-aPAH) involves obtaining a Doppler echocardiogram. Once the diagnosis is confirmed by right heart catheterization, SLE-aPAH patients are generally treated with oxygen, anticoagulants, and vasodilators. Although the prognosis and therapeutic responsiveness of these patients have improved with the addition of intensive immunosuppressive therapies, these treatments are still largely unproven. Recent data put the one-year survival rate for SLE-aPAH patients at 94%. Pregnant women are most at risk of dying due to undiagnosed SLE-aPAH, and screening should be considered essential in this population.
机译:肺动脉高压(PAH)通常与结缔组织疾病(CTD)相关,包括系统性硬化症和系统性红斑狼疮(SLE)。 SLE中PAH的患病率估计为0.5%至17.5%。 PAH的病理生理学涉及多种机制,从血管炎和​​原位血栓形成到间质性肺纤维化,这会增加肺血管阻力,并可能导致右心衰竭。免疫和炎症机制可能在CTD患者PAH的发病机理或进展中起重要作用,从而确立了抗炎和免疫抑制疗法的作用。 SLE中PAH的主要预测因素是Raynaud现象,抗U1RNP抗体和抗心磷脂抗体阳性。对怀疑与SLE相关的PAH(SLE-aPAH)的患者进行的一线诊断测试包括获取多普勒超声心动图。一旦右心导管检查证实了诊断,通常会用氧气,抗凝剂和血管扩张药治疗SLE-aPAH患者。尽管通过加强免疫抑制疗法可改善这些患者的预后和治疗反应,但这些疗法仍未得到充分证实。最新数据显示,SLE-aPAH患者的一年生存率为94%。孕妇由于未诊断为SLE-aPAH而死亡的风险最大,应考虑在这一人群中进行筛查。

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